Idiopathic systemic capillary leak syndrome (Clarkson's disease) presenting with recurrent hypovolemic shock
Davies, K.; Thomas, K.; Barton, L.; Williams, C.; Aujayeb, A.; Premchand, N.
Acute Medicine 20(1): 74-77
2021
ISSN/ISBN: 1747-4892 PMID: 33749696 Document Number: 18200
A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson's Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.
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